KEGG   DISEASE: ゾリンジャー・エリソン症候群
エントリ  
H01522                                                             
名称    
ゾリンジャー・エリソン症候群
概要    
Zollinger-Ellison syndrome (ZES) is a rare endocrinopathy caused by tumors of the pancreas and duodenum. These tumors, called gastrinomas, release gastrin to produce large amounts of acid that result in severe gastroesophageal peptic ulcer disease and diarrhea. Most ZES cases are sporadic, but about over 20 percent are caused by an inherited genetic disorder called multiple endocrine neoplasia type 1 (MEN1) [DS:H00247]. The clinical presentation is not specific for this disease and there is overlap of symptoms similar to those of a peptic ulcer. The most common symptoms include abdominal pain and diarrhea, sometimes accompanied by heartburn, nausea, and weight loss. Peptic ulceration complicated by bleeding is present in 25% of patients, and is more frequently in patients with sporadic ZES than in those with MEN1. In addition, the gastrinomas may be cancerous. The cancer can be spread to other parts of the body, most commonly to regional lymph nodes and the liver. The treatment of the ZES includes surgical removal and medical management of gastric acid hypersecretion for the prevention of malignant transformation and the genesis of complications.
カテゴリ  
内分泌代謝疾患
階層分類  
ヒト疾患 [BR:jp08402]
 内分泌代謝疾患
  その他の内分泌代謝疾患
   H01522  ゾリンジャー・エリソン症候群
ICD-11 による疾患分類 [BR:jp08403]
 05 内分泌, 栄養, 代謝の疾患
  内分泌疾患
   その他のグルコース調節または膵内分泌の疾患
    5A43  ガストリンの異常分泌
     H01522  ゾリンジャー・エリソン症候群
病因遺伝子 
MEN1 [HSA:4221] [KO:K14970]
治療薬   
シメチジン [DR:D00295]
ラニチジン塩酸塩 [DR:D00673]
ファモチジン [DR:D00318]
ロキサチジン酢酸エステル塩酸塩 [DR:D01467]
オメプラゾール [DR:D00455]
オメプラゾールナトリウム [DR:D05261]
ラベプラゾールナトリウム [DR:D00724]
エソメプラゾールマグネシウム水和物 [DR:D01984]
オクトレオチド酢酸塩 [DR:D02250]
リンク   
ICD-11: 5A43.1
ICD-10: E16.4
MeSH: D015043
OMIM: 131100
文献    
  著者
Epelboym I, Mazeh H
  タイトル
Zollinger-Ellison syndrome: classical considerations and current controversies.
  雑誌
Oncologist 19:44-50 (2014)
DOI:10.1634/theoncologist.2013-0369
文献    
PMID:9215689 (MEN1)
  著者
Agarwal SK, Kester MB, Debelenko LV, Heppner C, Emmert-Buck MR, Skarulis MC, Doppman JL, Kim YS, Lubensky IA, Zhuang Z, Green JS, Guru SC, Manickam P, Olufemi SE, Liotta LA, Chandrasekharappa SC, Collins FS, Spiegel AM, Burns AL, Marx SJ
  タイトル
Germline mutations of the MEN1 gene in familial multiple endocrine neoplasia type 1 and related states.
  雑誌
Hum Mol Genet 6:1169-75 (1997)
DOI:10.1093/hmg/6.7.1169
文献    
  著者
Gibril F, Schumann M, Pace A, Jensen RT
  タイトル
Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature.
  雑誌
Medicine (Baltimore) 83:43-83 (2004)
DOI:10.1097/01.md.0000112297.72510.32
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